Phenylketonuric diet negatively impacts on butyrate production

Phenylalanine (Phe) restricted diet, combined with Phe-free l-amino acid supplementation, is the mainstay of treatment for phenylketonuria (PKU). Being the diet a key factor modulating gut microbiota composition, the aim of the present paper was to compare dietary intakes, gut microbiota biodiversit...

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Published in:Nutrition, metabolism, and cardiovascular diseases metabolism, and cardiovascular diseases, 2018-04, Vol.28 (4), p.385-392
Main Authors: Verduci, E., Moretti, F., Bassanini, G., Banderali, G., Rovelli, V., Casiraghi, M.C., Morace, G., Borgo, F., Borghi, E.
Format: Article
Language:English
Subjects:
Adolescent
Adolescent Nutritional Physiological Phenomena
Bacteria - metabolism
Butyrate
Butyrates - metabolism
Case-Control Studies
Child
Child Nutritional Physiological Phenomena
Child, Preschool
Diet, Protein-Restricted - adverse effects
Dietary Carbohydrates - administration & dosage
Dietary Carbohydrates - metabolism
Faecalibacterium prausnitzii
Fatty Acids - metabolism
Feces - chemistry
Feces - microbiology
Fermentation
Gastrointestinal Microbiome
Gastrointestinal Tract - microbiology
Glycemic Index
Glycemic Load
Humans
Male
Nutritional Status
Nutritive Value
Phenylketonuria
Phenylketonurias - blood
Phenylketonurias - diet therapy
Phenylketonurias - microbiology
Phenylketonurias - physiopathology
Recommended Dietary Allowances
Short chain fatty acids
Treatment Outcome
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Summary:Phenylalanine (Phe) restricted diet, combined with Phe-free l-amino acid supplementation, is the mainstay of treatment for phenylketonuria (PKU). Being the diet a key factor modulating gut microbiota composition, the aim of the present paper was to compare dietary intakes, gut microbiota biodiversity and short chain fatty acids (SCFAs) production in children with PKU, on low-Phe diet, and in children with mild hyperphenylalaninemia (MHP), on unrestricted diet. We enrolled 21 PKU and 21 MHP children matched for gender, age and body mass index z-score. Dietary intakes, including glycemic index (GI) and glycemic load (GL), and fecal microbiota analyses, by means of denaturing gradient gel electrophoresis (DGGE) and Real-time PCR were assessed. Fecal SCFAs were quantified by gas chromatographic analysis. We observed an increased carbohydrate (% of total energy), fiber and vegetables intakes (g/day) in PKU compared with MHP children (p = 0.047), as well a higher daily GI and GL (maximum p 
ISSN:0939-4753
1590-3729
DOI:10.1016/j.numecd.2018.01.004